Article
Os odontoideum in wolcott-rallison syndrome: a case series of 4 patients.
Orphanet journal of rare diseases - 10 Feb 2016
Dias R P, Buchanan C R, Thomas N, Lim S, Solanki G, Connor S E J, Barrett T G, Kapoor R R
Abstract excerpt
Wolcott-Rallison Syndrome is the commonest cause of neonatal diabetes in consanguineous families. It is associated with liver dysfunction, epiphyseal dysplasia, and developmental delay. It is caused by mutations in eukaryotic translation initiation factor 2-α kinase 3 (EIF2AK3).We report 4 children with WRS and Os Odontoideum resulting in significant neurological compromise. This cervical spine abnormality has...
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